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The 2018 Zülch Prize: Autoimmunity in neurological diseases

Jerome Posner, Angela Vincent and Josep Dalmau have uncovered new diseases and have improved outcomes for neurological deficits

September 17, 2018

Cancer, infections and other still undescribed factors can trigger the body’s immune system. In some patients this results in an autoimmune attack against healthy tissue such as cells of the nervous system. This can lead to deficits such as memory loss, seizures, movement disorders, muscle weakness, and psychosis. The Gertrud Reemtsma Foundation has now paid tribute to Jerome Posner, Angela Vincent and Josep Dalmau for their research into how autoimmunity produces these neurological disorders that include paraneoplastic and non-paraneoplastic neurologic syndromes and the antibody-mediated autoimmune encephalitis syndromes. Their work has been pivotal in ensuring that many of these disorders are now recognised and patients promptly treated offering the best opportunity for neurologic improvement. In recognition of their outstanding achievements, the researchers have been awarded the Gertrud Reemtsma Foundation's K. J. Zülch Prize worth 50,000 euro. The award ceremony will take place in Cologne, Germany on the 21th of September 2018.

Medical practitioners have long been aware that autoimmune brain diseases can occur in patients with cancer. The scientists have made crucial advances in research into these immune diseases by providing detailed clinical descriptions and through the development of simple diagnostic tests, allowed practitioners to quickly recognize, diagnose, and treat these patients. Their work has also provided insights into how cancers and other triggers such as viral infections initiate the autoimmune attack. This has opened new avenues to research into how to prevent and optimally treat these diseases. For some patients, recognition of the autoimmune neurologic symptoms leads to the detection of a previously undetected cancer, allowing early cancer treatment and an increased chance of cure.

These diseases were largely unknown to the public but gained attention when in 2011 a polar bear named Knut drowned in his pool at the Berlin Zoological Garden. An autopsy revealed that one of the antibody-mediated autoimmune encephalitis syndromes known as anti-NMDA receptor encephalitis caused his unexpected demise. Then in 2012, a patient with this same disease wrote a best-selling novel about her experience.

The American Jerome Posner is considered a pioneer in the field of research into these syndromes and their causes, having systematically described many of the paraneoplastic diseases for the first time and developing the first blood tests used to diagnose these disorders. His former colleague from Barcelona, Spain, Josep Dalmau is known for discovering several autoimmune encephalitis syndromes, developing diagnostic tests, and revealing the mechanisms whereby the autoantibodies are directly responsible for the brain dysfunction.

British-born Angela Vincent initially studied autoimmune diseases of the peripheral nervous system in which signal transmission between nerves and muscles are impaired. This led her to the then revolutionary insight that certain antibodies can also attack the central nervous system. She subsequently developed the diagnostic methods widely used in hospitals today.

The Prize Winners

For many years, Jerome Posner was Head of Neurooncology at the Memorial Sloan-Kettering Cancer Center in New York and Professor of Neurology and Neuroscience at Cornell University in New York. Following his medical studies in Barcelona, Josep Dalmau initially worked as a neurologist before continuing his scientific career in Jerome Posner’s laboratory. He currently holds professorships at the University of Pennsylvania and the University of Barcelona.

Angela Vincent, a medically-qualified neuroimmunologist, worked at University College in London with the late Ricardo Miledi, and the Royal Free Hospital with the late John Newsom-Davis. She spent the last 30 years at the University of Oxford where until recently she ran a national and international diagnostic antibody service and research group. She currently holds honorary positions in Oxford and London.

The Zülch Prize

The K. J. Zülch Prize 2018 will be awarded at 10 o’clock on the 21th of September 2018 in the Hansasaal (main hall) at Cologne's historic City Hall. Following the laudations by Uwe Schlegel and Thomas Münte, Angela Vincent will talk about her research into autoimmune diseases of the peripheral and central nervous system. Following the laudation by Mathias Bähr, Josep Dalmau will talk about the discovery of anti-NMDA receptor encephalitis.